Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan.
Division of Pediatric Surgery, Department of Surgery, Aichi Medical University, Nagakute, Japan.
Lancet Gastroenterol Hepatol. 2017 Aug;2(8):610-618. doi: 10.1016/S2468-1253(17)30002-X.
Pancreaticobiliary maljunction is a congenital malformation in which the pancreatic and bile ducts join outside the duodenal wall, usually forming a long common channel. Because the action of the sphincter of Oddi does not regulate the function of the pancreaticobiliary junction in patients with pancreaticobiliary maljunction, two-way regurgitation occurs. Reflux of pancreatic juice into the biliary tract is associated with a high incidence of biliary cancer. Biliary carcinogenesis in patients with pancreaticobiliary maljunction is thought to follow the hyperplasia, dysplasia, then carcinoma sequence due to chronic inflammation caused by pancreatobiliary reflux. Pancreaticobiliary maljunction is diagnosed when an abnormally long common channel is evident on imaging studies. Congenital biliary dilatation involves both local dilatation of the extrahepatic bile duct, including the common bile duct, and pancreaticobiliary maljunction. Extrahepatic bile duct resection is the standard surgery for congenital biliary dilatation. However, complete excision of the intrapancreatic bile duct and removal of stenoses of the hepatic ducts are necessary to prevent serious complications after surgery.
胰胆管合流异常是一种先天性畸形，其中胰管和胆管在十二指肠壁外汇合，通常形成一个长的共同通道。由于Oddi 括约肌的作用不能调节胰胆管合流异常患者的胰胆管功能，会发生双向反流。胰液反流进入胆道与胆道癌的高发率有关。胰胆管合流异常患者的胆道癌变被认为是由于胰胆反流引起的慢性炎症导致的增生、发育不良，然后是癌序列。当影像学研究显示异常长的共同通道时，即可诊断胰胆管合流异常。先天性胆管扩张症涉及肝外胆管的局部扩张，包括胆总管和胰胆管合流异常。肝外胆管切除术是先天性胆管扩张症的标准手术。然而，为了防止手术后出现严重并发症，需要完全切除胰内胆管并清除肝内胆管狭窄。
